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Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with thrombotic states including elevated coagulation factor VIII (FVIII). Pulmonary endarterectomy (PEA) is the main treatment for CTEPH, and efficient anticoagulation is essential to prevent thromboembolism recurrence after surgery. We aimed to characterize longitudinal changes in FVIII and other coagulation biomarkers after PEA., Methods: Coagulation biomarker levels were measured at baseline and up to 12 months after operation in 17 consecutive patients with PEA. Temporal patterns of coagulation biomarkers, and correlation of FVIII with other coagulation biomarkers, were analyzed., Results: Baseline FVIII levels were elevated in 71% of the patients (mean 216 ± 67 IU/dl). FVIII doubled 7 days after PEA, peaking at 471 ± 87 IU/dl, and gradually returned to respective baseline levels within 3 months. Postoperative fibrinogen levels were also elevated. Antithrombin decreased at 1 to 3 days, D-dimer increased at 1 to 4 weeks, and thrombocytosis was observed at 2 weeks., Conclusions: FVIII is elevated in most patients with CTEPH. After PEA, early but transient elevation of FVIII and fibrinogen, and delayed reactive thrombocytosis, occurs, and warrants careful postoperative anticoagulation to prevent thromboembolism recurrence.

Chronic myelocytic leukemia (CML) can occasionally occur after long-term chemotherapy for solid tumors; solid tumors secondary to chemotherapy and biotherapy for CML have also been reported. However, concurrence of these two phenomena in an untreated patient has seldom been reported. Herein, we describe the case of a female patient in her early 60 s who was transferred to the liver surgery department after the discovery of a large liver mass and elevated plasma alpha-fetoprotein levels. She was initially diagnosed with liver cancer. Blood tests indicated an increased platelet count (2464 × 10 9 /L). Chromosomal examination from a bone marrow biopsy indicated the presence of the t(9;22) translocation, and subsequent fluorescence in situ hybridization and PCR were positive for the BCR-ABL rearrangement. A diagnosis of CML was made. The patient received hydroxyurea and imatinib to treat CML and underwent subsequent platelet-lowering therapy and a liver biopsy, which suggested moderately poorly differentiated adenocarcinoma or potentially hepatic metastatic carcinoma. However, the patient refused further pathological examination or screening for the site of the primary tumor. She died 6.5 months after discharge. The exact relationship between the two tumors remains unclear, and more patients need to be evaluated.

Antiphospholipid syndrome (APS) and myeloproliferative neoplasms (MPN) are associated with an increased risk of thrombosis. The optimal management of patients with coexistent APS and MPN has not been defined. A single centre and systematic literature review of patients with coexistent APS and MPN was performed. Cases were divided into two groups based on whether they met international consensus criteria for APS. Of the 12 studies identified, eight were excluded (leaving five of a total 54 patients), as although antiphospholipid antibodies (aPL) were documented, the diagnosis of APS was not conclusively demonstrated. Another ten patients with definite APS were identified at our centre. Fifteen patients (ten females, five males) were therefore included in this analysis (eleven definite APS and four highly likely), median age 44 (range: 13-71) years. Nine had polycythaemia vera and six, essential thrombocythaemia. Thirteen of the 15 patients (86.7%) had thrombotic APS (seven with initial venous events and six arterial) and two (13.3%) had obstetric APS. Nine patients were single-positive, and six double-positive for aPL. None were triple aPL-positive. Four patients at our centre had recurrent thrombotic/obstetric events, including while on anticoagulation/antiplatelet treatment.

Objective: To analyse the frequency and characteristics of the Janus kinase 2 ( JAK2) V617F mutation in patients with cerebral venous sinus thrombosis (CVST) with thrombocytosis., Methods: The study enrolled CVST patients with thrombocytosis that had undergone JAK2 V617F mutation detection to determine the frequency of the JAK2 V617F mutation in this cohort. Correlations between patient demographics, whole blood cell counts, targeted sequencing results and JAK2 V617F mutation status were determined., Results: A total of 23 patients were enrolled in the study: 11 (47.8%) with the JAK2 V617F mutation and 12 (52.2%) without the JAK2 V617F mutation. The mean platelet count was significantly higher in patients with the JAK2 V617F mutation than in patients without the mutation (478.1 ± 107.4 × 10 9 /l versus 374.4 ± 54.1 × 10 9 /l, respectively). There were no significant differences in age, sex, white blood cell count or haemoglobin level between the two groups. Other than single nucleotide polymorphisms, no hot-spot mutations associated with myeloid tumours other than the JAK2 V617F mutation were detected in four CVST patients that underwent targeted sequencing., Conclusion: The JAK2 V617F mutation was frequently detected in CVST patients with thrombocytosis and it was associated with higher platelet counts.

Thrombocytopenia is an important medical condition to understand prior to performing procedures in the foot and ankle. We have set forth to highlight factors a physician should take into consideration before performing procedures in the thrombocytopenic patient. A retrospective cohort analysis at a large academic institution was undertaken utilizing a cohort discovery tool to discover incidence and management strategies for patients with foot-related conditions that require in-office procedures. We demonstrate that a full history and physical are important to guide treatment along with complete blood count testing prior to intervention. We included all patients at the institution that underwent a foot and ankle procedure in-office with podiatric surgery over 10 years where thrombocytopenia was demonstrable via complete blood count within 3 months of the procedure. Patients' charts were reviewed for 1 year following podiatric intervention and outcomes were recorded. The cohort reveals that patients with thrombocytopenia have many advanced comorbidities but performing procedures in this cohort is safe. Complications from procedures included erythrocyte transfusion, ulcer recurrence, need for formal surgical intervention, infection, falls, and death. We then provide a brief discussion about the etiology and management options available for thrombocytopenia.

We describe an unusual presentation of toe ulceration in a 39-year-old otherwise healthy man. The left fifth toe was painful for several months with ulceration at the tip. The other toes appeared cyanotic and discolored. Foot pulses were normal. Laboratory testing revealed the platelet count at 1208 thousand/microliter, with hemoglobin 19.2g/dl and hematocrit 57%. The erythropoietin level was found to be markedly decreased at 1 mU/ml. The patient was given the diagnosis of polycythemia vera with iron deficiency. Phlebotomy was performed and aspirin and cytoreductive therapy with hydroxyurea was prescribed with resolution of the toe ulceration.

Several clinical studies have shown that thrombocytosis is a poor prognostic factor in some types of cancer, but data about the impact of thrombocytosis on prognosis in patients with colon cancer are very limited. We investigated the prevalence and prognostic effect of pre-operative thrombocytosis, defined as a platelet count > 400 x 10(9)/l, retrospectively in patients with node-negative colon cancer. Out of 198 patients, 24 (12.1%) had thrombocytosis, and its presence correlated with tumour depth and lymphatic invasion. Univariate analysis revealed that disease-free survival and overall survival were shorter in patients with pre-operative thrombocytosis than those without thrombocytosis. On multivariate analysis, thrombocytosis alone retained significance as a poor prognostic factor for both disease-free survival and overall survival. In conclusion, this study shows an association between thrombocytosis and poor survival in patients with node-negative colon cancer. The preoperative platelet count may help to identify patients with an unfavourable prognosis in this subgroup.

Compared to primary thrombocytosis such as that caused by essential thrombocytosis, reactive thrombocytosis is generally regarded as benign. However, reactive thrombocytosis has infrequently been reported to cause severe and even fatal complications. Two fatal cases of reactive thrombocytosis and iron deficiency anemia associated with peripheral/pulmonary vascular and cerebrovascular thrombosis are described. The literature on thrombosis and reactive thrombocytosis associated with iron deficiency anemia is reviewed.

Background: Pyoderma gangrenosum is an uncommon ulcerative condition associated with inflammatory bowel disease, arthritis, and hematologic disease. We report a patient with essential thrombocythemia and pyoderma gangrenosum., Objective: This article is a review of the associations between pyoderma gangrenosum and other diseases., Results: There have been two previous reports of patients with pyoderma gangrenosum and essential thrombocythemia., Conclusion: There may be a possible association between pyoderma gangrenosum and essential thrombocythemia. The diagnosis of pyoderma gangrenosum should be considered in patients with essential thrombocythemia and cutaneous ulcers.

Low-risk essential thrombocythemia patients include patients aged 18 to < 80 years with no vascular risk factor or previous thrombosis, no associated disease, a normal life expectancy, and a platelet count between 400 and 1,000 x 10(9)/L up to 1,500 x 10(9)/L. Asymptomatic essential thrombocythemia patients may be at risk for microvascular circulation disturbances. The indication for low-dose aspirin in asymptomatic essential thrombocythemia patients is uncertain, therefore randomization for aspirin 50 mg versus placebo is recommended. Symptomatic essential thrombocythemia patients with erythromelalgia and its ischemic complications, atypical transient ischemic attacks, minor stroke, visual disturbances and "superficial thrombophlebitis" in the absence of bleeding, vascular risk factors, or vascular disease have a clear indication for aspirin in a regular dose. To determine whether 50 mg/day is as effective as 100 mg/day for the prophylaxis of microvascular circulation disturbances in essential thrombocythemia, a randomized trial comparing low-dose aspirin 50 mg versus 100 mg at platelet counts between 400 and 1,000 up to 1,500 x 10(9)/L is recommended. To address the question whether reduction of the platelet count to normal (< 350 x 10(9)/L) is as effective as low-dose aspirin for the long-term relief of microvascular circulation disturbances, a randomized study comparing low-dose aspirin with the correction of platelet count to normal by anagrelide is recommended. High-risk essential thrombocythemia patients have a clear indication for platelet reductive therapy, including: (a) platelets > 1,500 x 10(9)/L, history of major thrombosis (myocardial infarction, stroke, peripheral occlusive vascular disease), or presence of vascular disease (e.g., arteriosclerosis); (b) history or presence of spontaneous or major bleedings, bleedings elicited by low-dose aspirin for the secondary prevention of vascular complications in essential thrombocythemia at platelet counts < 1500 x 10(9)/L, and side effects of long-term aspirin treatment such as gastritis; and progression from low- to high-risk essential thrombocythemia patients during follow-up or progressive myeloproliferative disease such as significant splenomegaly, myelofibrosis, leukocytosis, etc. To address the question of optimal treatment of high-risk essential thrombocythemia patients, randomization for anagrelide versus interferon at < 65 years of age and anagrelide versus hydroxyurea at an age > 65 years is recommended.

The clinical course of a case of primary thrombocythemia is described, complicated by a variety of recurrent and sequential thrombotic manifestations, involving the peripheral as well as the cerebrovascular and coronary microvasculature, demonstrating that thrombocythemia should be considered as an important risk factor for thrombosis, already at platelet count states in excess of 400 x 10(9)/L.

On the basis of clinical, laboratory, and histopathologic studies, the authors discern three distinct types of red, congested, and burning extremities that need to be distinguished for effective treatment according to their etiology: erythromelalgia in thrombocythemia, primary erythermalgia, and secondary erythermalgia. Each entry is discussed in turn.

The observation that Philadelphia chromosome-positive acute lymphoblastic leukemia (ALL) may progress to chronic myelogenous leukemia (CML) has been well-documented among adult patients but reported only rarely in children. In this report, we describe a pediatric patient with atypical ALL, who subsequently went on to develop classical adult-type CML. This patient is unique and important because of the unusual clinical findings that she exhibited during the "ALL" phase, namely, extreme thrombocytosis, marrow fibrosis, and persistent splenomegaly, which heralded the onset of frank CML. It is suggested that the patient with presumed, but atypical, ALL be carefully evaluated for CML presenting in lymphoblastoid crisis, and that all patients with the morphologic diagnosis of ALL undergo complete cytogenetic and immunologic marker studies to confirm the diagnosis.

Although thrombotic occlusion of small vessels in thrombocytosis is allegedly well recognized, it has been reported only rarely. We treated 4 patients with primary thrombocytosis in whom ischemic toe lesions with normal peripheral pulses were the presenting and only symptoms. The symptoms preceded diagnosis by 5 years, 3 years, 1 year, and 1 month respectively. Treatment of the hematologic disorder resulted in disappearance of the toe lesions. Thrombocytosis should be routinely searched for in patients with ischemic toes and normal peripheral pulses.

A retrospective study of thrombocytosis in children with Hemophilus influenzae meningitis was performed. Forty-five percent of patients had a platelet count greater than 500 X 10(9)/l during hospitalization. The mean platelet count was 284 X 10(9)/l on admission, and gradually increased to 648 X 10(9)/l on day 11 of hospitalization. These results document that thrombocytosis is common among children with H. influenzae meningitis and suggest that it is probably a recovery phenomenon.